title: Long-term evolution of neuropsychological competences in encephalopathy with status epilepticus during sleep: a variable prognosis.
abstract: OBJECTIVE
  The aim of this study was to evaluate the long-term cognitive outcome in children with continuous spikes and waves during slow wave sleep (CSWS syndrome).
  
  
  METHODS
  We reviewed the neuropsychological tests of 25 children diagnosed with CSWS between 1987 and 2010 and with a mean follow-up of 13.5 years.
  
  
  RESULTS
  Cognitive performances worsened during CSWS in virtually all patients. Seven patients (28%) with nonlesional epilepsy had a positive outcome; three patients (12%) showed persistence of motor deficit without involvement of cognitive functions; and seven patients (28%) who presented a long duration of CSWS (mean = 28.1 months) had a negative cognitive outcome. In 6 patients (24%) with structural or metabolic disorders before CSWS onset cognitive outcomes did not change; 2 patients (8%) had a negative outcome irrespective of the duration or presence of other neurologic disorders before CSWS onset. Forty-four percent of children with CSWS demonstrated permanent cognitive impairment.
  
  
  CONCLUSIONS
  The long-term outcome of CSWS syndrome is variable and seems to depend on treatment response, disease duration, and underlying etiology.
authors.affiliation: National Neurologic Institute "C. Mondino," Child Neuropsychiatry Unit, University of Pavia, Pavia, Italy.
authors.forename: Maria C
authors.initials: MC
authors.lastname: Pera
authors.forename: Daniela
authors.initials: D
authors.lastname: Brazzo
authors.forename: Nausicaa
authors.initials: N
authors.lastname: Altieri
authors.forename: Umberto
authors.initials: U
authors.lastname: Balottin
authors.forename: Pierangelo
authors.initials: P
authors.lastname: Veggiotti
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009483:Neuropsychological Tests; D011379:Prognosis; D012893:Sleep Wake Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology*; D013226:Status Epilepticus / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: CSWS; ESES; Epilepsy; Neuropsychological evaluation; Outcome